Correlation between prognosis and response to treatment in children with FSGS

Mitra Naseri, MD. Pediatric nephrologists, Mashhad University of Medical Sciences

Nephrology department

Dr. sheikh children’s hospital

Abas Madani MD. Pediatric Nephrologist

Nephrology department, Tehran University of Medical Sciences

Pediatric Medical center, Dr Gharib St, Tehran, Iran

Nematolah Attaii MD. Pediatric Nephrologist

Nephrology department, Tehran University of Medical Sciences

Pediatric Medical center, Dr Gharib St, Tehran, Iran

Abstract:

Objective: To determine prognostic value of response to treatment in patients with focal segmental glomerulo-sclerosis.

Introduction: FSGS includes 10-15% of idiopathic Nephrotic syndrome in children.

Bulk of evidence supports disease relationship with immune system. Unfortunately, responses to immunosuppressive drugs are not desirable and progression to end-stage renal disease is common.

Materials and Methods: We analyzed 62 out of 99 cases of biopsy proven idiopathic FSGS who were followed for at least 5-years or till renal failure occurred during study. Study design was historical cohort and patients were divided into two groups: exposed (resistant to treatment) and non-exposed (responsive to treatment).

Correlation between prognosis and response to treatment was statistically evaluated. P-value £ 0.05 and relative risk ³ 1 were considered significant.

Results: In 3 out of 25 steroid responsive patients (12%) and 22 out of 37 steroid resistant patients (59.5%), disease progressed to renal failure.

Disease progressed to renal failure in 2 out of 11 cyclophosphamide responsive patients (18.1%), 17 out of 23 cyclophosphamide resistant patients (74.3%), and 8 out of 14 cyclosporine resistant patients (57.1%). 2 patients who responded to cyclosporine had normal renal function at the time of the last follow up.

Conclusion: We concluded that favorable response to steroid and cyclophosphamide treatment is a protective factor against disease progression to end stage renal disease and resistance to these drugs implies a poor prognosis.

For making any definite conclusion concerning response to cyclosporine treatment and prognosis, similar studies with a larger sample are required.

Keywords: children, Nephrotic syn, FSGS, steroid responsive, steroid resistant, prognosis